Acute rupture of primary hepatic angiosarcoma with Kasabach-Merritt syndrome: a rare case report and literature review.

BACKGROUND: Primary hepatic angiosarcoma (PHA) is an extremely rare malignant tumor originating from vascular endothelial cells, accounting for only 0.1-2% of primary liver malignancies. Kasabach-Merritt syndrome (KMS) is a rare complication characterized by thrombocytopenia, coagulation dysfunction, and microangiopathic hemolytic anemia, seldom reported in adult patients with hepatic angiosarcoma.

CASE PRESENTATION: We describe a 74-year-old female with a history of vinyl chloride exposure who presented with acute right upper quadrant pain, shock, and multiple ecchymoses, scattered petechiae. Laboratory tests revealed thrombocytopenia and coagulation abnormalities. Imaging demonstrated a large ruptured hepatic mass with hemoperitoneum. The patient underwent emergency right hemihepatectomy, with pathology confirming hepatic angiosarcoma. Postoperatively, the patient's coagulation parameters normalized, confirming resolution of KMS. No recurrence was noted at the 9-month follow-up.

CONCLUSION: Acute rupture of hepatic angiosarcoma with KMS is a life-threatening condition requiring rapid multidisciplinary intervention. Emergency hepatectomy not only controlled the hemorrhage but also removed the lesion causing KMS. Clinicians should maintain a high index of suspicion for hepatic angiosarcoma in patients presenting with spontaneous hepatic hemorrhage and coagulation abnormalities.